Tripathy SR
Background: Fibrous dysplasia (FD) is an abnormal bony lesion of unknown aetiology with insufficient bone maturation and fibrous tissue replacement. It can affect any bone but maxilla is most commonly affected facial bone. Affection of craniofacial bones with skull base involvement, though rare, is important for neurosurgeons. Orbital invasion is very rare. Diagnosis is late due to nonspecific symptoms, low suspicion and relative rarity. Polyostotic FD grows very rapidly and is frequently associated with deformities, complications and recurrence.
Case report: A 35 year old male presented with painful tender swelling, chronic discharging sinus, skin defect and foul smelling discharge over the previous operated site of left fronto - orbital region. He also had 2 episodes of generalised tonic clonic convulsions. He was operated for left frontal FD 15 yrs back. The current CT scan revealed a solid lesion in the left fronto-ethmoido-orbital region extending to skull base with left frontal lobar compression. He underwent a two stage surgery; first for surgical excision of diseased bone and after 1 month for filling the bony defect with gracillis muscle free vascular graft (FGMVG), anatomising with the superficial temporal vessels. Biopsy confirmed disorganized and immature bone. Patient’s symptoms got resolved with improvement of the facial deformity.
Conclusion: Craniofacial fibrous dysplasia involving orbit and skull base is a very rare osteopathy. Re-do surgery may be required even after an interval of one and a half decade. In extensive literature research, ROFFD treated with FGMVG couldn’t be traced. So this may be a first such case report; adding to the armamentarium of the Neurosurgeons and craniofacial surgeons in the fight against this dreaded disease.
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